References

Huntington's Disease Association. What causes Huntington's disease?. https://www.hda.org.uk/huntingtons-disease/what-is-huntingtons-disease/what-causes-huntingtons-disease (accessed June 2022)
Roos RA. Huntington's disease: a clinical review. Orphanet J Rare Dis. 2010; 5 https://doi.org/10.1186/1750-1172-5-40
Medline Plus. National Library of Medicine (US). 2020. https://ghr.nlm.nih.gov/condition/huntington-disease (accessed June 2022)
Myers RH. Huntington's disease genetics. NeuroRx. 2004; 1:255-262 https://doi.org/10.1602/neurorx.1.2.255
McCusker EA, Loy CT. Huntington Disease: The complexities of making and disclosing a clinical diagnosis after premanifest genetic testing. Tremor Other Hyperkinet Mov (N Y). 2017; 7 https://doi.org/10.7916/D8PK0TDD
Nold CS. Huntington disease. JAAPA. 2017; 30:46-47 https://doi.org/10.1097/01.JAA.0000512243.43624.33
Nopoulos PC. Huntington disease: a single-gene degenerative disorder of the striatum. Dialogues Clin Neurosci. 2016; 18:91-98 https://doi.org/10.31887/DCNS.2016.18.1/pnopoulos
Evans SJ, Douglas I, Rawlins MD Prevalence of adult Huntington's disease in the UK based on diagnoses recorded in general practice records. J Neurol Neurosurg Psychiatry. 2013; 84:1156-1160 https://doi.org/10.1136/jnnp-2012-304636
Wexler NS, Collett L, Wexler AR Incidence of adult Huntington's disease in the UK: a UK-based primary care study and a systematic review. BMJ Open. 2016; 6 https://doi.org/10.1136/bmjopen-2015-009070
Veenhuizen RB, Kootstra B, Vink W Coordinated multidisciplinary care for ambulatory Huntington's disease patients. Evaluation of 18 months of implementation. Orphanet J Rare Dis. 2011; 6 https://doi.org/10.1186/1750-1172-6-77
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Huntington's disease: the role of dentistry in the multidisciplinary team

From Volume 49, Issue 7, July 2022 | Pages 577-582

Authors

Emma Critchley

BDS, MFDS (Edin), DSCD, MSc (Rest), MSCD (Edin)

Speciality Registrar Special Care Dentistry, North West Deanery, Lancashire Teaching Hospitals NHS Foundation Trust, Chorley and South Ribble Hospital

Articles by Emma Critchley

Finbar O'Mahony

BDS, MFDS (Glas), DSCD, MSCD (Glas)

Consultant in Special Care Dentistry, Lancashire Teaching Hospitals NHS Foundation Trust, Chorley and South Ribble Hospital

Articles by Finbar O'Mahony

Elizabeth Lemon

Specialist Speech and Language Therapist, Adult Community SALT Ribbleton Clinic, Preston, Lancashire and South Cumbria NHS Foundation Trust

Articles by Elizabeth Lemon

Angela Adkins

BDS, FDSRCPS, MSNDRCS

Consultant Special Care Dentistry, LTHTR, UK

Articles by Angela Adkins

Abstract

Huntington's disease is a progressive, genetic disorder, leading to changes to cognition, movement and behaviour. The oral health of these patients can rapidly deteriorate if they are not assessed and managed early. This article explores the role of the dental team, as part of the multidisciplinary team, in caring for Huntington's disease patients. It highlights some of the challenges in providing oral care for this complex group, along with a pathway of care, used by one Special Care Dental Team, in helping to reduce oral complications.

CPD/Clinical Relevance: It is important for GDPs to understand the oral health needs of patients with Huntington's disease and their role in supporting the oral health of these patients.

Article

Huntington's disease (HD) is a hereditary, neurodegenerative disorder caused by a mutation in the huntingtin gene,1,2 found on chromosome 4.3 This mutated gene affects the production of an important protein required by neurones in the central nervous system.1,2 HD is inherited in an autosomal dominant pattern,3 which means any children of HD gene carriers have a 50% chance of inheriting the mutated gene.4

The classic sign of HD is chorea (involuntary, jerky movements) that gradually progresses to all muscles;2,3 however, it is not only muscle movement that is affected, and changes in cognition and behaviour are also seen.1 Early signs and symptoms can include irritability, depression, poor coordination, slight involuntary movements and problems with short-term memory.1,3,5 As the disease advances, these involuntary movements become more pronounced, and walking, talking and swallowing become increasingly problematic.2 Cognitive abilities decline and many patients develop dementia.6 Over the course of the disease, daily activities, such as tooth brushing, cleaning and eating, become more challenging and patients become more dependent.2

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